婴儿和儿童期严重新皮质癫痫综合征的临床表现.doc

该文献由北京癫痫病医院（）整理Infants and children with severe neocortical epilepsy syndrome clinical manifestation(婴儿和儿童期严重新皮质癫痫综合征的临床表现)Typically, the involvement of children in the prior to the onset of normal. Usually with focal motor seizures, partial status epilepticus, generalized tonic-clonic seizures ( GTCS ) or with automatism in complex partial seizures onset. WON continuous state may occur in 20% of patients. In 50% of cases the blood ring attack in the previous 6 months, with upper respiratory tract infections, otitis media or tonsillitis history.典型的，受累儿童在发病前正常。通常以局灶性运动性发作、部分性癫痫持续状态、全面性强直阵挛发作GTCS)或不伴自动症的复杂部分性发作起病。WON持续状态可能出现于20%的患者中。50%的病例在血响发作前6个月左右，有上呼吸道感染、中耳炎或扁桃体炎史。When the disease, focal motor seizures involve the distal muscles ( a thumb, finger, mouth or eyes ), but as time advances, the affected area spread to adjacent areas, having a walk and a more diffuse, and sleep in the persistence of. Along with the progress of. There are a variety of seizures, seizure frequency, and gradually increased during episodes of prolonged, often accompanied by seizures after hemiplegia. Partial epilepsy to state in 60% patients, and can last for several days, often mixed with laterality tonic-clonic seizures, but also can develop as a generalized tonic-clonic seizure. Appears in episodes after initial hemiparesis, short duration, but gradually develop persistent. In addition, there are reported in the literature in histologically confirmed Kozhevvikov Rssmussen syndrome cases, but without OAR attack.发病时，局灶性运动性发作主要累及远端少数肌群(拇指、手指、嘴角或眼角)，但是随着时间进展，受累部位扩散至周围相邻区域，具有游走性和更加弥散，并且在睡眠中也持续存在。随着病程的进展。出现多种发作，并且发作频率逐渐增加、发作期延长，经常伴有发作后偏瘫。部分性癫痫待续状态见于60%的患者，并可以持续数天，经常混有偏侧强直一阵挛发作，也可发展为全面性强直一阵挛发作。出现于发作后的偏瘫，最初持续时间短暂，但逐步发展为持久性。另外，也有文献报进了经组织学确诊的Kozhevvikov一Rssmussen综合征病例，但是不伴有OAR发作。The clinical course is divided into three stages临床病程分三个阶段The first stage : the key to simple motion or somatosensory as feature. The situation is relatively rare to partial status epilepticus, not with automatism in complex partial seizures or secondary GTCS onset, may also represent the seizure type combination. After a few weeks or months, gradually become frequent episodes.第一阶段:主要以简单运动或躯体感觉发作为特征。比较少见的情况是以部分性癫痫持续状态、不伴自动症的复杂部分性发作或继发性GTCS起病，也可能表现为这些发作类型的组合。经过数周或数月，发作逐渐地变得频繁。Second stage: episodes of deterioration and the intact hemisphere neurological symptoms progress to feature. This phase usually begins about the occurrence of seizures after 3 months, but can also be delayed until the disease was first symptoms after 10 years. In this phase, attacks become more frequent, the affected area is more extensive. Episodes of longer duration. At the same time, as the disease progression in children with sharp, lasting psychomotor defects, including associated with cognitive and language impaired ( aphasia and dysarthria ) of hemiparesis, hemihypesthesia, hemianopia. Most of the brain damage occurred in 8 after a 12 month.第二阶段:以发作的恶化和受累半球的神经系统症状进展为特征。这一阶段通常大约开始于出现癫痫发作3个月后，但也可延迟至该病首次症状后10年之久。在此阶段，发作变得更加频繁，受累部位更加广泛。发作持续时间更长。同时，随着病情急剧进展，患儿出现持久的精神运动缺陷，包括伴有认知和语言受损(失语和构音障碍)的轻偏瘫、偏侧感觉减退、偏盲。大部分脑损害发生于起病后的8一12个月。Progressive neurological and psychomotor function degradation, either with or without delusion seizure deterioration, Koehevnikov is a typical Rssmusrxn syndrome.进行性神经系统和精神运动功能退化，无论伴有或不伴有痴痫发作的恶化，是Koehevnikov一Rssmusrxn综合征的典型特征。The third stage: the disease showed the frequency of seizures an